AB0598 SYSTEMIC LUPUS ERYTHEMATOSUS WITH CEREBRAL HEMORRHAGE DUE TO AUTOIMMUNE ACQUIRED COAGULATION FXIII/13 FACTOR DEFICIENCY: A CASE STUDY AND LITERATURE REVIEW
Authors: A. Aoki, H. Kobayashi
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Autoimmune acquired coagulation FXIII/13 factor deficiency (AiF13D) is a rare bleeding disorder characterized by the formation of autoantibody to F13. The treatment of AiF13D requires immunosuppressive (IS) therapy in addition to hemostatic therapy. Japanese guidance recommends high-dose glucocorticoid (GC) as initial therapy while cyclophosphamide (CPA), rituximab (RIT), and ciclosporin (CYA) are administered in refractory cases. Ichinose reported that 16 of 93 (17%) AiF13D patients had autoimmune diseases (AIDs) such as systemic lupus erythematosus (SLE) [1]. The guidance recommends that IS therapy for patients with underlying AIDs should be selected based on treatment guidelines for AIDs; however, recommendations for the choice of IS drugs are unavailable. <h3>Objectives</h3> To report the case of our patient and to analyze the reported cases of SLE complicated AiF13D. <h3>Methods</h3> We searched PubMed, Google Scholar, and Ichushi Web for articles or abstracts written in English and Japanese using the following terms: [acquired FXIII/13 factor deficiency or inhibitors] and SLE. <h3>Results</h3> A 24-year-old woman from Southeast Asia presented to the emergency department with headache and nausea; a hematoma was noted in the right temporal lobe on head CT. Her PT, APTT, platelet count, and bleeding time were normal. She developed impaired consciousness and underwent an emergency craniotomy to remove the hematoma. During surgery, no abnormal blood vessels were noted in the brain, and the hematoma was removed. However, recurrent hemorrhages occurred on days 2 and 13 after hospitalization, and she underwent reoperation. On day 18, F13 activity was found to be less than 3%, and an F13 preparation (Fibrogamin P) was administered. A positive cross-mixing test with normal plasma was positive for the F13 inhibitor, and a diagnosis of AiF13D was made [2]. In addition, she was diagnosed with SLE based on positive antinuclear and anti-dsDNA antibodies (Ab), hypocomplementemia, and lymphopenia. PSL 50 mg (1mg/kg/day) was prescribed. Owing to the local infection after cranioplasty, no IS drugs were used. hydroxychloroquine (HCQ) and belimumab were added, while tapering PSL. During 1 year period, anti-dsDNA Ab decreased and F13 activity remained above 40% without administering Fibrogamin P (Figure 1). The reported 11 cases of SLE complicated AiF13D and this case are summarized in the Table 1. IS therapy included CPA, CPA, and RIT besides GC; belimumab was administered only in our case. <h3>Conclusion</h3> The prognosis of patients with AiF13D is poor. Even after weaning from acute hemorrhage, long-term IS therapy is often necessary and death by infection is common. Based on our experience, we suggest belimumab as a therapy option for SLE complicated AiF13D. <h3>References</h3> [1]Ichinose A, et al. Blood Reviews 2017; 31: 37-45. [2]Ichinose A, et al. Thromb Haemost 2016; 116:772-774. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.